background: factor xiii (fxiii) is a heterotetramer composing two subunits including fxiii-a and fxiii-b. several common gene variations were observed in fxiii-a gene with obvious ethnic difference. this study assessed pattern of tyr204phe as a common fxiii-a gene variation among iranian population. materials and methods: this study was conducted on eighty iranian unrelated individuals. genotyp...

factor xiii deficiency (fxiiid) is an extremely rare hemorrhagic disorder with a prevalence of 1/3-5 million. management of disease is performed by fresh frozen plasma (ffp), cryoprecipitate (cp) or fxiii concentrate (fibrogammin p®). our objective was to assess safety and effectiveness of fibrogammin p® in patients with fxiiid. for this purpose we designed this long-term follow up study on a l...

background congenital factor xiii (fxiii) deficiency is a rare severs autosomal recessive bleeding disorder. objectives the aim of the study was to determine the c559t > c fxiiia genotype frequency in patients with fxiii hemophilia who lived in sistan and balouchestan province in southeast of iran. patients and methods we determined the genotype of 180 patients with factor xiii hemophilia by te...

Factor XIII or "fibrin-stabilizing factor," is a transglutaminase circulates in the blood circulation as a hetero tetramer with two catalytic A subunits and two carrier B subunits. This important coagulation factor has a crucial role in clotting cascade and produces strong covalent bonds between soluble formed fibrin monomers during coagulation. This stable cross linked fibrin strands are resis...

background: iran has a large group of patients with severe congenital factor xiii deficiency (fxiiid) and trp187arg mutation that is most disease causing mutation of fxiii in the world is only observed in southeast of iran with 352 patients with fxiiid. 743 patients with fxiiid was observed in 17 provinces of iran but tehran city with more than 12 million population has no any registered patien...

congenital factor xiii deficiency is a very rare bleeding disorder, but because of the high rate of consanguineous marriages, it is common in sistan and baluchestan province of iran. the discovery of promoter hypermethylation of numerous mirnas in human diseases has demonstrated an epigenetic mechanism for aberrant mirna expression. the present study has analyzed methylation and expression stat...

stroke, though rare in children is among the top 10 causes of death in childhood. incidence of ischemic and hemorrhagic stroke is the same in children .we report a case of hemorrhagic stroke in a two year old girl who presented with a limp, inability to stand on the left leg and left hemiparesia. her complaint began 10 days ago after a bout of left clonic seizure. she had been admitted to the h...

conclusions the case was fully investigated and diagnosed as a case with factor xiii deficiency causing such uncontrolled bleeding after extraction of a tooth. introduction factor xiii deficiency is a relatively rare hereditary bleeding disorder, which is usually found during infancy or early childhood. this condition may involve both genders within different races in an equal manner. its incid...

Background: Congenital factor XIII (FXIII) deficiency is one of the rarest bleeding disorders with a prevalence of one per 2 million in the general population. The disorder is accompanied by a high rate of life-threatening bleeding. Due to normal results of routine coagulation tests, diagnosis of the disorder is challenging, but molecular methods can be used for precise diagnosis. Direct mutati...